放线菌素D治疗肾母细胞瘤致肝窦阻塞综合征1例并文献复习  

Sinusoidal obstruction syndrome after Actinomycin treatment in Wilms tumor—a case report and review of the literature

作　　者：钟笛箫[1] 张朝霞[1] 胡梦泽[1] 张蕾[1] 曲东[2] 刘嵘[1] ZHONG Dixiao;ZHANG Zhaoxia;HU Mengze;ZHANG Lei;QU Dong;LIU Rong(Department of Hematology and Oncology;Department of Critical Care Medicine, Children′s Institute of Pediatrics affiliated Children′s Hospital.)

机构地区：[1]首都儿科研究所附属儿童医院血液内科,北京100020 [2]首都儿科研究所附属儿童医院重症医学科,北京100020

出　　处：《中国小儿血液与肿瘤杂志》2020年第4期220-225,共6页Journal of China Pediatric Blood and Cancer

摘　　要：目的了解肾母细胞瘤(WT)在辅助化疗中发生肝窦阻塞综合征/肝小静脉闭塞病(SOS/VOD)的情况。方法回顾性分析我科收治的1例Ⅱ期WT患儿,应用放线菌素D(Act-D)化疗后出现SOS/VOD的临床资料,并复习相关文献。结果WT患儿术后化疗d5出现进行性血小板减少,化疗d9逐渐出现发热、肝大、腹水、黄疸,符合SOS/VOD的临床诊断,予暂停化疗、血浆置换、抗感染、对症支持等综合治疗,d22痊愈。结论Act-D治疗WT患儿有一定几率引发SOS/VOD,早期出现孤立性血小板减少可作为SOS/VOD即将发生的警示。Objective To report the clinical features,treatment and follow-up of a patients with Wilms′Tumor(WT)presented sinusoidal obstruction syndrome/veno-occlusive disease(SOS/VOD)during the adjuvant chemotherapy.Methods A retrospective analysis was performed in a one-year-old WT(stageⅡ)patient in our hospital who complicated with SOS/VOD after standard dose of Act-D.The clinical data were analyzed and related literatures were reviewed.Results The WT patient developed thrombocytopenia on day 5 after a single dose of Act-D combined chemotherapy of vindesine.The patient presented fever,hepatomegaly,ascites and jaundice on day 9,and diagnosed as SOS/VOD.The patient was withdrew chemotherapy and treated with plasma exchange,antibiotics,and support treatments.The patient discharged with complete recovery after 22-day treatment in hospital.Conclusions SOS/VOD may occur in WT patients after Act-D chemotherapy.Isolated thrombocytopenia following Act-D is a warning sign of imminent SOS/VOD.

关 键 词：肝窦阻塞综合征/肝小静脉闭塞病 放线菌素D 肾母细胞瘤 

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